Wild-type (senile) amyloidosis medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Roukoz A. Karam, M.D.[2]
Overview
Medical Therapy
- There is no treatment for wild-type (senile) amyloidosis; the mainstay of therapy is supportive treatment aimed at symptoms of the disease.
- Since wild-type (senile) amyloidosis commonly presents with symptoms of right-sided heart failure and carpal tunnel syndrome, treating amyloid deposits in the heart is important.[1]
- Supportive treatment is with diuretics, antiarrhythmics or pacemaker implantation, anticoagulation where supraventricular arrhythmias are present, and an avoidance of digoxin and calcium channel blockers.
- Antihypertensives are usually poorly tolerated as these patients can be profoundly hypotensive.
- For some patients with severe wild-type (senile) amyloidosis, heart transplant may be the best option.
- Pharmacologic therapies aimed at stabilizing the transthyretin molecule and thus preventing amyloid formation are being actively investigated.
- In patients with transthyretin amyloid cardiomyopathy, Tafamidis was associated with reductions in all-cause mortality and cardiovascular-related hospitalizations and reduced the decline in functional capacity and quality of life as compared with placebo.[2]
References
- ↑ Tanskanen M, Peuralinna T, Polvikoski T, Notkola IL, Sulkava R, Hardy J; et al. (2008). "Senile systemic amyloidosis affects 25% of the very aged and associates with genetic variation in alpha2-macroglobulin and tau: a population-based autopsy study". Ann Med. 40 (3): 232–9. doi:10.1080/07853890701842988. PMID 18382889.
- ↑ Maurer MS, Schwartz JH, Gundapaneni B, Elliott PM, Merlini G, Waddington-Cruz M; et al. (2018). "Tafamidis Treatment for Patients with Transthyretin Amyloid Cardiomyopathy". N Engl J Med. 379 (11): 1007–1016. doi:10.1056/NEJMoa1805689. PMID 30145929.